[Neuroendocrine carcinoma of the skin (Merkel cell tumor)].
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Biomedical subjects
Publications and source records attributed to G Mauduit.
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A 51-year-old mason presented with a large tumour on his scalp which had developed over the previous 2 years. Histological examination showed the presence of large vessels with muscular coats some of which appeared to be venules and others arterioles. They had markedly swollen cuboidal endothelial cells and a surrounding mononuclear cell infiltrate which in areas formed lymphoid follicles some of which had germinal centers. There was no evidence of tissue or blood eosinophilia. The unusual clinical and histological features of this case are emphasized and the nosology of this rare condition is discussed.
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Pseudo-scleroderma should not be confused with true scleroderma, the prognosis of which is unpredictable and often serious. Progressive acrosclerosis must be differentiated from Raynaud's disease, congenital or hereditary disorders of unknown aetiology: Werner's syndrome, acrogeria and progeria; Rothmund-Thomson's syndrome, Steinert's disease, phenylketonuria, disorders of glycogen metabolism; metabolic disorders: mutilating acropathies, scleromyxoedema, porphyria cutanea tarda; occupational and iatrogenic disorders: acroosteolysis, toxic epidermic syndrome (Spain), scleroderma-like change induced by bleomycin, chronic graft-versus-host disease; and leprosy. Acute diffuse scleroderma should not be confused with Buschke's scleroedema, sclerema neonatorum, systemic amyloidosis and scleroderma-like changes in hypothyroidism. Linear pseudo-scleroderma is suggested by the following scleroderma-like conditions: facial hemiatrophy, acrodermatitis atrophicans, melorheostosis, pseudo-scleroderma after corticosteroid injection, and cutaneous lesions in carcinoid syndrome. Scleroderma in plaque must be differentiated from hypodermitis sclerotisans, panatrophy and localized lipoatrophies, hypodermitis after vitamin K injection, basal cell carcinoma, necrobiosis lipoidica, vitiligo, chronic radiodermatitis, cutaneous lymphatic invasion. Scleroderma-like changes after drug injection (vitamin B12, progestin), anetoderma barely resemble morphea guttata.
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A 69-year-old man developed a Hodgkin's disease 2 years after he started a mycosis fungoides. He presented cutaneous plaques of mycosis fungoides. The first signs of Hodgkin's disease was acquired ichthyosis and loss of weight. Echotomography of the abdomen showed retroperitoneal nodes. A laparotomy was performed and the histopathologic examination of the lymph nodes revealed a Hodgkin's disease type 2 (sclero-nodular). The liver and the bone marrow were involved. A chemotherapy was completed but the patient died 10 months later. The review of the literature showed 24 patients with Hodgkin's disease and mycosis fungoides or Sézary syndrome. Relation between mycosis fungoides, Hodgkin's disease and lymphomatoid papulosis are discussed.
Retinoids are used in the treatment of lichen planus (LP) disease, but their mechanism of action remains unknown. We studied the epidermal Langerhans' cells in four patients with chronic LP, before and after treatment with aromatic retinoids. This study uses a new technique of quantification and exploits two monoclonal antibodies-OKT 6 specific for Langerhans' cells and BL 2 specific for HLA-DR antigens. Our results show an increase in the number of dendritic epidermal cells after treatment with oral retinoids. The number of OKT 6-positive cells is greater than the number of BL 2-positive (HLA-DR) cells before and after treatment.
TP5, a synthetic pentapeptide corresponding to thymopoietin 32-36, was administered alone to eight adult volunteer patients with sarcoidosis. A dose of 50 mg of TP5 was given iv, three times a week for 6 weeks, to three patients with erythema nodosum (EN) and bilateral hilar adenopathy, and to one patient with sarcoids of the skin; and for 12 weeks, to the other four patients with skin sarcoids. Before treatment and every 3 weeks thereafter clinical features; routine laboratory tests; tests for cellular immunity, humoral immunity, and auto immunity; IgE levels; and polymorphonuclear functions were recorded. EN disappeared in 3 weeks; hilar adenopathy improved or disappeared more slowly. Improvement of skin sarcoids was noted (lesions flattened or were cured). No side effects were observed. No evident changes in routine tests, humoral and auto immunity, IgE levels, and functions of polymorphonuclear leukocytes were observed. Cutaneous anergy to skin multi-tests was observed in seven patients before treatment, and was corrected with TP5 in six cases. In patients with low levels of peripheral blood T cells and suppressor T cells (as determined using specific monoclonal antibodies), a progressive normalization was obtained with TP5. These data support the efficacy of TP5 in sarcoidosis, although the action of the drug may be only temporary, as spontaneous remission may have occurred in this open trial of only eight cases.
The purpose of this study was to examine the phenotype of the cutaneous immunocompetent cells and Langerhans cells in malignant and benign tumour infiltrates (basal cell and squamous cell carcinomas, malignant melanoma, seborrheic keratosis and naevus) by the use of monoclonal antibodies directed against T cell populations and Langerhans cells. This in situ investigation indicates that the lymphocytes participating in the inflammatory reaction around skin tumours are mainly of the cytotoxic/suppressor class. It suggests, moreover, that interaction occurs in the skin between T lymphocytes and HLA-DR suppressing cells. The in situ study of the inflammatory immune response should be a useful complement to in vitro investigations in the exploration of immune reaction against tumours of the skin.
Biopsies from twenty-nine malignant skin tumours and four benign dermatoses and fourteen samples of normal skin were stained for cell surface membrane or cytoplasmic expression of HLA heavy chain and light chain (beta 2 microglobulin) by use of the immunoperoxidase technique on frozen sections using monoclonal antibodies. Results showed a loss of beta 2 microglobulin from the surface of malignant cells in twenty-four out of twenty-nine cutaneous malignancies and the presence of HLA heavy chain in all twenty-nine primary and secondary tumours. Expression of both heavy and light chains was observed in the four benign lesions and in normal epidermis in all samples examined. This would appear to be the first report of dissociation of heavy and light HLA chain expression in various forms of cutaneous malignancy.
The purpose of the present study was to examine the phenotype of the immunocompetent cells in cutaneous infiltrates and peripheral blood in Lichen Planus. 14 patients have been studied using Monoclonal Antibodies directed against T cell populations. We have used in this study the indirect immunofluorescence technique. Helper cells (OKT4+) and Suppressor/cytotoxic cells (OKT8+) have been observed in all cutaneous infiltrates, and numerous Langerhans cells identified by OKT6 in epidermis and dermis. In peripheral blood, the balance between the two major T cell subsets (OKT4+ and OKT8+) was increased by a reduction of the percentage of Suppressor/cytotoxic cells. This balance is statistically significant (P 0,02). Our results confirm the existence of a lymphocytotoxic process in Lichen Planus.
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Peripheral blood lymphocytes frm 16 patients with sarcoidosis (9 patients with skin sarcoids, 7 patients with erythema nodosum and bilateral hilar adenopathy) and cutaneous anergy and from 23 age-matched healthy controls were characterized by reactivity with monoclonal antibodies OKT3, OKT4, OKT8 directed to surface antigens of T lymphocytes, helper-inducer and suppressor-cytotoxic T cell subsets, respectively. In contrast to healthy controls, patients with sarcoidosis had reduced percentages of OKT3+, OKT4+ and OKT8+ cells and a major decrease in the OKT8+ (suppressor) subset. However, these changes were significant only in the group of patients with acute sarcoidosis (erythema nodosum). This abnormal T cell distribution correlates with the alterations in cell-mediated immunity previously observed and suggests the presence of a defective circulating suppressor T cell activity in acute sarcoidosis.